RESUMEN
Arrhythmogenic cardiomyopathy (ACM) is a familial heart disease characterized by cardiac dysfunction, arrhythmias, and myocardial inflammation. Exercise and stress can influence the disease's progression. Thus, an investigation of whether a high-fat diet (HFD) contributes to ACM pathogenesis is warranted. In a robust ACM mouse model, 8-week-old Desmoglein-2 mutant (Dsg2mut/mut) mice were fed either an HFD or rodent chow for 8 weeks. Chow-fed wildtype (WT) mice served as controls. Echo- and electrocardiography images pre- and post-dietary intervention were obtained, and the lipid burden, inflammatory markers, and myocardial fibrosis were assessed at the study endpoint. HFD-fed Dsg2mut/mut mice showed numerous P-wave perturbations, reduced R-amplitude, left ventricle (LV) remodeling, and reduced ejection fraction (%LVEF). Notable elevations in plasma high-density lipoprotein (HDL) were observed, which correlated with the %LVEF. The myocardial inflammatory adipokines, adiponectin (AdipoQ) and fibroblast growth factor-1, were substantially elevated in HFD-fed Dsg2mut/mut mice, albeit no compounding effect was observed in cardiac fibrosis. The HFD not only potentiated cardiac dysfunction but additionally promoted adverse cardiac remodeling. Further investigation is warranted, particularly given elevated AdipoQ levels and the positive correlation of HDL with the %LVEF, which may suggest a protective effect. Altogether, the HFD worsened some, but not all, disease phenotypes in Dsg2mut/mut mice. Notwithstanding, diet may be a modifiable environmental factor in ACM disease progression.
Asunto(s)
Dieta Alta en Grasa , Animales , Dieta Alta en Grasa/efectos adversos , Ratones , Modelos Animales de Enfermedad , Miocardio/patología , Miocardio/metabolismo , Fibrosis , Masculino , Remodelación Ventricular , Desmogleína 2/genética , Miocarditis/etiología , Miocarditis/fisiopatología , Ratones Endogámicos C57BL , Displasia Ventricular Derecha Arritmogénica/etiología , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Adiponectina/sangre , Inflamación , Cardiomiopatías/etiología , Cardiomiopatías/fisiopatologíaAsunto(s)
Displasia Ventricular Derecha Arritmogénica , Ablación por Catéter , Humanos , Displasia Ventricular Derecha Arritmogénica/cirugía , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Ablación por Catéter/métodos , Endocardio/cirugía , Endocardio/fisiopatología , Masculino , Electrocardiografía , Persona de Mediana Edad , Femenino , AdultoRESUMEN
OBJECTIVES: Arrhythmogenic cardiomyopathy (ACM) is a complex cardiac disorder associated with ventricular arrhythmias. Understanding the relationship between mechanical uncoupling and cardiac structural changes in ACM patients is crucial for improved risk stratification and management. METHODS: In this study, we enrolled 25 ACM patients (median age 34 years, 72% men) based on the 2019 Modified Task Force and Padua criteria. Patients were categorized by the presence or absence of clinically relevant ventricular tachycardia (crVT), necessitating emergency interventions. Right ventricular-arterial coupling (VAC) was assessed using echocardiography. Low-rank regression splines were employed to model left ventricular ejection fraction (LVEF) and right ventricular ejection fraction (RVEF) in relation to VAC. RESULTS: Positive associations were observed between VAC and LVEF (ρ = 0.472, p = 0.023), RVEF (ρ = 0.522, p = 0.038), and right ventricular (RV) indexed stroke volume (ρ = 0.79, p < 0.001). Patients with crVT exhibited correlations with RV shortening, reduced RVEF (39.6 vs. 32.2%, p = 0.025), increased left ventricular (LV) mass (38.99 vs. 45.55, p = 0.045), and LV end-diastolic volume (LVEDV) (56.99 vs. 68.15 mL/m2, p = 0.045). Positive associations for VAC were noted with LVEDV (p = 0.039) and LV mass (p = 0.039), while negative correlations were observed with RVEF by CMR (p = 0.023) and RV shortening by echocardiography (p = 0.026). CONCLUSIONS: Our findings underscore the significance of right VAC in ACM, demonstrating correlations with RV and LVEF, RV stroke volume, and clinically relevant arrhythmias. Insights into RVEF, LV mass, and end-diastolic volume provide valuable contributions to the understanding of ACM pathophysiology and may inform risk assessment strategies.
OBJETIVOS: La miocardiopatía arritmogénica (MCA) es un trastorno cardíaco complejo asociado con arritmias ventriculares (AV). Comprender la relación entre el desacoplamiento mecánico y los cambios estructurales cardíacos en pacientes con MCA es crucial para una estratificación de riesgos y una gestión mejorada. MÉTODOS: En este estudio, reclutamos a 25 pacientes con MCA (edad media 34 años, 72% hombres) basándonos en los criterios del Task Force 2019 y los criterios de Padua. Los pacientes se clasificaron según la presencia o ausencia de taquicardia ventricular clínicamente relevante (crVT), que requería intervenciones de emergencia. Se evaluó el acoplamiento ventricular derecho-arterial (VAC) mediante ecocardiografía. Se utilizaron low-rank regression splines para modelar la fracción de eyección del ventrículo izquierdo (FEVI) y la fracción de eyección del ventrículo derecho (FEVD) en relación con el VAC. RESULTADOS: Se observaron asociaciones positivas entre el VAC y la FEVI (ρ = 0.472, p = 0.023), la FEVD (ρ = 0.522, p = 0.038) y el volumen de eyección indexado del ventrículo derecho (ρ = 0.79, p < 0.001). Los pacientes con crVT mostraron correlaciones con acortamiento del ventrículo derecho, disminución de la FEVD (39.6 vs. 32.2%, p = 0.025), aumento de la masa ventricular izquierda (38.99 vs. 45.55, p = 0.045) y volumen diastólico final del ventrículo izquierdo (VDVI) (56.99 vs. 68.15 mL/m2, p = 0.045). Se observaron asociaciones positivas para el VAC con el VDVI (p = 0.039) y la masa ventricular izquierda (p = 0.039), mientras que se observaron correlaciones negativas con la FEVD por RMC (p = 0.023) y el acortamiento del ventrículo derecho por ecocardiografía (p = 0.026). CONCLUSIONES: Nuestros hallazgos subrayan la importancia del VAC derecho en la MCA, demostrando correlaciones con la FEVD y FEVI, el volumen de eyección del ventrículo derecho y arritmias clínicamente relevantes. Las percepciones sobre la FEVD, la masa ventricular izquierda y el volumen diastólico final proporcionan contribuciones valiosas para comprender la fisiopatología de la MCA y pueden informar estrategias de evaluación de riesgos.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Volumen Sistólico , Humanos , Masculino , Femenino , Adulto , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Volumen Sistólico/fisiología , Persona de Mediana Edad , Ecocardiografía/métodos , Imagen por Resonancia Magnética/métodos , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/diagnóstico por imagen , Taquicardia Ventricular/etiología , Prueba de Estudio Conceptual , Adulto Joven , Función Ventricular Derecha/fisiología , Función Ventricular Izquierda/fisiologíaAsunto(s)
Displasia Ventricular Derecha Arritmogénica , Mitocondrias Cardíacas , Humanos , Mitocondrias Cardíacas/metabolismo , Mitocondrias Cardíacas/patología , Animales , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/metabolismo , Displasia Ventricular Derecha Arritmogénica/genética , Miocitos Cardíacos/metabolismo , Miocitos Cardíacos/patologíaRESUMEN
BACKGROUND: Although the epicardial predominance of substrate abnormalities has been well demonstrated in early stages of arrhythmogenic right ventricular cardiomyopathy (ARVC), endocardial (ENDO) ablation may suffice to eliminate ventricular tachycardia (VT) in some patients. OBJECTIVES: This study aimed to report the long-term outcomes of ENDO-only ablation in ARVC patients and factors that predict VT-free survival. METHODS: We included consecutive patients with Task Force Criteria diagnosis of ARVC undergoing a first ENDO-only VT ablation between 1998 and 2020. Ablation was predominantly guided by activation/entrainment mapping for mappable VTs and pace mapping/targeting abnormal electrograms for unmappable VTs. The primary endpoint was freedom from any recurrent sustained VT after the last ENDO-only ablation. RESULTS: Seventy-four ARVC patients underwent ENDO-only VT ablation. VT noninducibility was achieved in 49 (66%) patients. During median follow-up of 6.6 years (Q1-Q3: 3.4-11.2 years), 40 (54.1%) patients remained free from any VT recurrence with rare VT ≤2 episodes in additional 12.2%. Among patients with noninducibility, VT-free survival was 75.5% during long-term follow-up. In multivariable analysis, >45 y of age at diagnosis (HR: 0.41; 95% CI: 0.17-0.98) and VT noninducibility (HR: 0.36; 95% CI: 0.16-0.80) were predictors of VT-free survival. CONCLUSIONS: Long-term VT-free survival can be achieved in over half of ARVC patients following ENDO-only VT ablation, increasing to over 75% if VT noninducibility is achieved. Our results support consideration of a stepwise ENDO-only approach before proceeding to epicardial ablation if VT noninducibility can be achieved particularly in older patients.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Ablación por Catéter , Endocardio , Taquicardia Ventricular , Humanos , Masculino , Displasia Ventricular Derecha Arritmogénica/cirugía , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/complicaciones , Femenino , Ablación por Catéter/métodos , Ablación por Catéter/estadística & datos numéricos , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/fisiopatología , Persona de Mediana Edad , Adulto , Endocardio/cirugía , Endocardio/fisiopatología , Resultado del Tratamiento , Recurrencia , Estudios RetrospectivosRESUMEN
BACKGROUND: Epicardial approach in ventricular tachycardia (VT) ablation is still regarded as a second-step strategy, due to the risk of complications. We evaluated the frequency that epicardial ablation targets were identified and ablation performed following pericardial access compared with unnecessary pericardial access for different VT causes and potential markers of epicardial VT. METHODS: All VT ablation procedures including epicardial approach over a 10-year period were included. First-line epicardial approach was indicated in arrhythmogenic right ventricular cardiomyopathy (ARVC) and postmyocarditis VT; in patients with idiopathic dilated cardiomyopathy (IDCM) and postmyocardial infarction, indications resulted from available imaging techniques or 12-lead VT morphology. The epicardial approach was considered useful if epicardial ablation was performed after epicardial mapping. Feasibility, complications, and long-term outcome were reported. RESULTS: Four hundred and eighty-eight subjects with a median age of 60 years (interquartile range, 47-65) and of left ventricle ejection fraction 41% (interquartile range, 30-55) underwent 626 epicardial VT ablations. Percutaneous access had a success rate of 92.2% and a complication rate of 3.6%. Overall, epicardial approach was, respectively, indicated to 11.8% of postmyocardial infarction patients, 49.5% in IDCM, 94% in myocarditis, and 90.7% in ARVC. Epicardial ablation at the first ablation attempt was performed in 9.3% of postmyocardial infarction patients, 28.8% in IDCM, 86.5% in myocarditis, and 81.3% in patients with ARVC. In first-line epicardial group, ARVC and myocarditis showed the highest odds for epicardial ablation (OR, 4.057 [95% CI, 1.299-8.937]; P=0.007; OR, 3.971 [95% CI, 1.376-11.465]; P=0.005, respectively). IDCM independently predicted unnecessary epicardial approach (OR, 2.7 [95% CI, 1.7-4.3]; P<0.001). After a follow-up of 41 months (interquartile range, 19-64), patients with IDCM experienced higher rate of recurrences and mortality compared with other causes. CONCLUSIONS: Epicardial approach is integral part of ablation armamentarium regardless of the VT cause, with high feasibility and low complication rate in experienced centers. Our data support its use at first ablation attempt in VTs related to ARVC and myocarditis.
Asunto(s)
Ablación por Catéter , Mapeo Epicárdico , Pericardio , Taquicardia Ventricular , Humanos , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/diagnóstico , Masculino , Persona de Mediana Edad , Ablación por Catéter/efectos adversos , Ablación por Catéter/métodos , Femenino , Resultado del Tratamiento , Anciano , Pericardio/cirugía , Pericardio/fisiopatología , Centros de Atención Terciaria , Factores de Tiempo , Estudios Retrospectivos , Estudios de Factibilidad , Displasia Ventricular Derecha Arritmogénica/cirugía , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Factores de Riesgo , Recurrencia , Cardiomiopatía Dilatada/cirugía , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/diagnósticoRESUMEN
Purpose To demonstrate the myocardial strain characteristics of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), based on revised Task Force Criteria (rTFC), and to explore the prognostic value of strain analysis in ARVC. Materials and Methods This retrospective study included 247 patients (median age, 38 years [IQR, 28-48 years]; 167 male, 80 female) diagnosed with ARVC, based on rTFC, between 2014 and 2018. Patients were divided into "possible" (n =25), "borderline" (n = 40), and "definite" (n = 182) ARVC groups following rTFC. Biventricular global strain parameters were calculated using cardiac MRI feature tracking (FT). The primary outcome was defined as a composite of cardiovascular events, including cardiovascular death, heart transplantation, and appropriate implantable cardioverter defibrillator discharge. Univariable and multivariable cumulative logistic regression and Cox proportional hazards regression analysis were used to evaluate the diagnostic and prognostic value of right ventricle (RV) strain parameters. Results Patients with definite ARVC had significantly reduced RV global strain in all three directions compared with possible or borderline groups (all P < .001). RV global longitudinal strain (GLS) was an independent predictor for disease (odds ratio, 1.09 [95% CI: 1.02, 1.16]; P = .009). During a median follow-up of 3.4 years (IQR, 2.0-4.9 years), 55 patients developed primary end point events. Multivariable analysis showed that RV GLS was independently associated with the occurrence of cardiovascular events (hazard ratio, 1.15 [95% CI: 1.07, 1.24]; P < .001). Kaplan-Meier analysis showed that patients with RV GLS worse than median had a higher risk of combined cardiovascular events (log-rank P < .001). Conclusion RV GLS derived from cardiac MRI FT demonstrated good diagnostic and prognostic value in ARVC. Keywords: MR Imaging, Image Postprocessing, Cardiac, Right Ventricle, Cardiomyopathies, Arrhythmogenic Right Ventricular Cardiomyopathy, Revised Task Force Criteria, Cardiovascular MR, Feature Tracking, Cardiovascular Events Supplemental material is available for this article. © RSNA, 2024.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Ventrículos Cardíacos , Humanos , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Masculino , Femenino , Persona de Mediana Edad , Adulto , Estudios Retrospectivos , Pronóstico , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/patología , Imagen por Resonancia Cinemagnética/métodos , Imagen por Resonancia Magnética , Función Ventricular Derecha/fisiologíaAsunto(s)
Displasia Ventricular Derecha Arritmogénica , Humanos , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Ecocardiografía/métodos , Masculino , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/fisiopatología , Femenino , Imagen por Resonancia Cinemagnética/métodosRESUMEN
INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an infrequent hereditary disorder distinguished by fibrofatty replacement of the myocardium in the right ventricular, which predisposes individuals to life-threatening arrhythmias. This case delineates an ARVC patient who suffered recurrent bouts of sustained ventricular tachycardia (VT). In this case, we mainly discuss the application of myocardial contrast echocardiography (MCE) in displaying myocardial fibrosis in patients with ARVC. CASE PRESENTATION: A 43-year-old male experienced three episodes of unexplained VT over an eight-year period, accompanied by symptoms of chest discomfort, palpitations and dizziness. Coronary angiography revealed no significant coronary stenosis. The electrocardiogram (ECG) results indicated characteristic epsilon waves in right precordial leads, and subsequent echocardiography identified right ventricular enlargement and right ventricular systolic dysfunction. MCE further disclosed regional myocardial ischemia at the epicardium of the left ventricular apex. Ultimately, cardiovascular magnetic resonance imaging (CMR) corroborated the ARVC diagnosis, highlighting linear intensification in the right ventricle during the delayed enhancement. CONCLUSION: Prompt identification of ARVC is crucial for timely intervention and management. MCE may offer an effective and valuable technique for the detection of myocardial involvement in ARVC patient.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Electrocardiografía , Taquicardia Ventricular , Humanos , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/terapia , Masculino , Adulto , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/etiología , Valor Predictivo de las Pruebas , Función Ventricular Derecha , Fibrosis , Ecocardiografía , Miocardio/patología , Frecuencia Cardíaca , Imagen por Resonancia CinemagnéticaRESUMEN
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition with a prevalence ranging from 1 in 2000 to 1 in 5000 individuals. ARVC is a significant contributor to sudden cardiac death, particularly in young individuals and athletes, and remains challenging to diagnose definitively. We conducted a single-center retrospective study to evaluate the presentations, electrocardiogram findings, and imaging characteristics of ARVC patients evaluated at our center between 2021 and 2023. Notably, our study is the second investigation of ARVC conducted in Pakistan. We report divergent symptom prevalence as compared to the current literature and have incorporated the Task Force Criteria. Despite limited access to cardiac magnetic resonance (CMR) facilities worldwide, our findings underscore the critical role ofCMR in ARVC diagnosis. Our cohort had a mortality rate of 17 % highlighting the importance of early detection and the need for improved diagnostic facilities for ARVC in the region.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Electrocardiografía , Imagen por Resonancia Cinemagnética , Humanos , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Estudios Retrospectivos , Masculino , Femenino , Adulto , Imagen por Resonancia Cinemagnética/métodos , Pronóstico , Pakistán/epidemiología , Persona de Mediana Edad , Adulto Joven , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/prevención & control , Prevalencia , AdolescenteRESUMEN
BACKGROUND: Desmin (DES) pathogenic variants cause a small proportion of arrhythmogenic cardiomyopathy (ACM). Outcomes data on DES-related ACM are scarce. OBJECTIVES: This study sought to provide information on the clinical phenotype and outcomes of patients with ACM caused by pathogenic variants of the DES gene in a multicenter cohort. METHODS: We collected phenotypic and outcomes data from 16 families with DES-related ACM from 10 European centers. We assessed in vitro DES aggregates. Major cardiac events were compared to historical controls with lamin A/C truncating variant (LMNA-tv) and filament C truncating variant (FLNC-tv) ACM. RESULTS: Of 82 patients (54% males, median age: 36 years), 11 experienced sudden cardiac death (SCD) (n = 7) or heart failure death (HFd)/heart transplantation (HTx) (n = 4) before clinical evaluation. Among 68 survivors, 59 (86%) presented signs of cardiomyopathy, with left ventricular (LV) dominant (50%) or biventricular (34%) disease. Mean LV ejection fraction was 51% ± 13%; 36 of 53 had late gadolinium enhancement (ring-like pattern in 49%). During a median of 6.73 years (Q1-Q3: 3.55-9.52 years), the composite endpoint (sustained ventricular tachycardia, aborted SCD, implantable cardioverter-defibrillator therapy, SCD, HFd, and HTx) was achieved in 15 additional patients with HFd/HTx (n = 5) and SCD/aborted SCD/implantable cardioverter-defibrillator therapy/sustained ventricular tachycardia (n = 10). Male sex (P = 0.004), nonsustained ventricular tachycardia (P = 0.017) and LV ejection fraction ≤50% (P = 0.012) were associated with the composite endpoint. Males with DES variants had similar outcomes to historical FLNC-tv and LMNA-tv controls. However, females showed better outcomes than those with LMNA-tv. In vitro experiments showed the characteristic finding of DES aggregates in 7 of 12 variants. CONCLUSIONS: DES ACM is associated with poor outcomes which can be predicted with potentially successful treatments, underscoring the importance of familial evaluation and genetic studies to identify at risk individuals.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Muerte Súbita Cardíaca , Desmina , Fenotipo , Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Muerte Súbita Cardíaca/etiología , Desmina/genética , Displasia Ventricular Derecha Arritmogénica/genética , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Adulto Joven , Desfibriladores Implantables , Trasplante de Corazón , AdolescenteRESUMEN
AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by progressive myocardial dysfunction and associated with an increased risk of major cardiovascular (CV) events. To determine right heart strain (ventricular and atrial global longitudinal strain (RVGLS and RAGLS) in patients with definite ARVC and its association with adverse events during follow-up. METHODS AND RESULTS: RVGLS and RAGLS were analysed in focused right heart apical views from 70 patients using TomTec ImageArena and association with a composite endpoint was determined (sustained ventricular arrhythmia and cardiovascular death). Over a median follow-up duration of 4.9 years, 26 (37%) patients met the endpoint. RVGLS was significantly impaired in the event group (-11.5 [-13.3 to -10.2] %) vs. the no-event group (-15.8 [-17.1 to -14.5] %, P < 0.001), and so was RAGLS (22.8 [21.4-27.4] % vs. 31.5 [25.1-39.6] %, respectively, P < 0.001). In Cox regression, RVGLS (HR 1.36, P < 0.001) and RAGLS (HR 0.92, P = 0.002) were associated with a higher risk of adverse events. In multivariable Cox regression models, RVGLS and RAGLS remained independent of and were incremental to age, gender, and conventional RV parameters, and model fitness was improved when RVGLS and RAGLS were applied together rather than alone. CONCLUSION: RVGLS and RAGLS are more impaired in patients with adverse events and associated with adverse events independent of age, gender, and conventional RV parameters. When RVGLS and RAGLS are applied together, prediction models are improved suggesting that right heart strain may form part of the echocardiographic routine protocol in patients with ARVC.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Humanos , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Masculino , Femenino , Persona de Mediana Edad , Adulto , Medición de Riesgo , Pronóstico , Estudios de Cohortes , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/fisiopatología , Estudios de Seguimiento , Imagen por Resonancia Cinemagnética/métodos , Estudios Retrospectivos , Modelos de Riesgos ProporcionalesRESUMEN
Arrhythmogenic Cardiomyopathy (AC), an inherited cardiac disorder characterized by myocardial fibrofatty replacement, carries a significant risk of sudden cardiac death (SCD) due to ventricular arrhythmias. A comprehensive multimodality imaging approach, including echocardiography, cardiac magnetic resonance imaging (CMR), and cardiac computed tomography (CCT), allows for accurate diagnosis, effective risk stratification, vigilant monitoring, and appropriate intervention, leading to improved patient outcomes and the prevention of SCD. Echocardiography is primary tool ventricular morphology and function assessment, CMR provides detailed visualization, CCT is essential in early stages for excluding congenital anomalies and coronary artery disease. Echocardiography is preferred for follow-up, with CMR capturing changes over time. The strategic use of these imaging methods aids in confirming AC, differentiating it from other conditions, tracking its progression, managing complications, and addressing end-stage scenarios.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Imagen Multimodal , Humanos , Imagen Multimodal/métodos , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Displasia Ventricular Derecha Arritmogénica/terapia , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Manejo de la Enfermedad , Imagen por Resonancia Cinemagnética/métodos , Ecocardiografía/métodos , Muerte Súbita Cardíaca/prevención & control , Muerte Súbita Cardíaca/etiologíaRESUMEN
OBJECTIVES: The present study aimed to investigate the incremental prognostic value of the right ventricular fractal dimension (FD), a novel marker of myocardial trabecular complexity by cardiac magnetic resonance (CMR) in patients with arrhythmogenic cardiomyopathy (ACM). METHODS: Consecutive patients with ACM undergoing CMR were followed up for major cardiac events, including sudden cardiac death, aborted cardiac arrest, and appropriate implantable cardioverter defibrillator intervention. Prognosis prediction was compared by Cox regression analysis. We established a multivariable model supplemented with RV FD and evaluated its discrimination by Harrell's C-statistic. We compared the category-free, continuous net reclassification improvement (cNRI) and integrated discrimination index (IDI) before and after the addition of FD. RESULTS: A total of 105 patients were prospectively included from three centers and followed up for a median of 60 (48, 66) months; experienced 36 major cardiac events were recorded. Trabecular FD displayed a strong unadjusted association with major cardiac events (p < 0.05). In the multivariable Cox regression analysis, RV maximal apical FD maintained an independent association with major cardiac events (hazard ratio, 1.31 (1.11-1.55), p < 0.002). The Hosmer-Lemeshow goodness of fit test displayed good fit (X2 = 0.68, p = 0.99). Diagnostic performance was significantly improved after the addition of RV maximal apical FD to the multivariable baseline model, and the continuous net reclassification improvement increased 21% (p = 0.001), and the integrated discrimination index improved 16% (p = 0.045). CONCLUSIONS: In patients with ACM, CMR-assessed myocardial trabecular complexity was independently correlated with adverse cardiovascular events and provided incremental prognostic value. CLINICAL RELEVANCE STATEMENT: The application of FD values for assessing RV myocardial trabeculae may become an accessible and promising parameter in monitoring and early diagnosis of risk factors for adverse cardiovascular events in patients with ACM. KEY POINTS: ⢠Ventricular trabecular morphology, a novel quantitative marker by CMR, has been explored for the first time to determine the severity of ACM. ⢠Patients with higher maximal apical fractal dimension of RV displayed significantly higher cumulative incidence of major cardiac events. ⢠RV maximal apical FD was independently associated with major cardiac events and provided incremental prognostic value in patients with ACM.
Asunto(s)
Displasia Ventricular Derecha Arritmogénica , Ventrículos Cardíacos , Humanos , Masculino , Femenino , Pronóstico , Persona de Mediana Edad , Displasia Ventricular Derecha Arritmogénica/diagnóstico por imagen , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Estudios Prospectivos , Fractales , Adulto , Imagen por Resonancia Magnética/métodos , Imagen por Resonancia Cinemagnética/métodos , Muerte Súbita CardíacaRESUMEN
BACKGROUND: Substrate-based ablation targets areas of delayed and fractionated electrograms during sinus rhythm, which are sensitive for identifying the ventricular tachycardia (VT) isthmus but is influenced by the activation wavefront direction and decremental pacing. OBJECTIVES: The aim of this study was to correlate the areas of latest activation during varying wavefront activation mapping and decremental pacing mapping with sites critical to the VT isthmus. METHODS: Three high-density electroanatomical substrate maps were created in patients presenting for ablation of monomorphic VT: 1) native sinus rhythm; 2) right ventricular (RV) apical pacing; and 3) an RV apical S2 map following the S1 drive train at 20 ms above the ventricular effective refractory period. Areas corresponding to the latest activation were compared with the VT isthmus identified by conventional mapping. RESULTS: Twenty patients with structural heart disease with a mean age of 55.6 ± 16.9 years were included. The majority of the cohort consisted of patients with ischemic heart disease (50%) and arrhythmogenic RV cardiomyopathy (35%). Epicardial ablation was performed in 45% of patients. The concordance of the site of latest activation in sinus rhythm with the VT isthmus was 75%. The location of the latest activation during RV apical pacing corresponded with the VT isthmus in 85% of cases. However, in 95% of cases, the site of the latest activation following the S2 stimulus colocalized to the VT isthmus. CONCLUSIONS: In a mix of underlying myocardial substrates, regions of conduction slowing during decremental pacing colocalize with the VT isthmus more frequently than sinus rhythm activation mapping and may have a role in substrate-based ablation where VT induction is undesirable.